systemic sclerosis and raynauds Flashcards

1
Q

scleroderma

A

skin thickening

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2
Q

which gender is it more common in

A

women

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3
Q

what do patients often present with in systemic sclerosis

A

raynauds

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4
Q

variations of systemic scleroiss

A

diffuse and limited

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5
Q

where does diffuse affect

A

above and below elbows and knees and face and trunk; whole body

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6
Q

what does limited affect

A

only below elbows and knees plus face

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7
Q

CREST of systemic sclerosis

A

CREST
calcinosis ; calcium deposits in skin
raynauds
esophageal dysfunciton; acid reflux
scleroderma; thickening of skin on hands and fingers
telangiectasis; dilation of capillaries causing red dots around face

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8
Q

causes of systemic sclerosis

A

genetic predisposition and environmental triggers

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9
Q

pathophysiology

A

result of vascular damage in the skin and organs

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10
Q

symptoms and signs

A

fatigue weakness
joint stiffness/pain
skin fibrosis presenting as thickened plaques
lack of wrinkles
tightenting of the skin around the mouth

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11
Q

unpleasant risk

A

pulmonary hypertension

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12
Q

what autoantibody is associated with pulmonary hypertension in limited ssc

A

anti centromere antibody - ACA

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13
Q

what autoantibody assoc with diffuse in progressive interstitial lung disease

A

anti topoisomerase SCL-7

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14
Q

non medical help

A
  • Avoid smoking
  • Gentle skin stretching to maintain the range of motion
  • Regular emollients
  • Avoiding cold triggers and wearing gloves for Raynaud’s
  • Physiotherapy to maintain healthy joints
    Occupational therapy for adaptations to daily living to cope with limitations
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15
Q

medical help for raynauds

A

oral vasodilators including ccbs and phosphodiesterase inhibitors
Iv vasodilators

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16
Q

immunomodulatory therapies for skin thickening

A

mycophenolate and methotrexate and cyclophosphamide

16
Q

what is raynauds

A

hands become white in cold weather due to vasospasm and vasoconstriction
fingers become pure white

17
Q

how do fingers become blue in raynauds

A

deoxygenated blood is floating around

18
Q

what happens to raynauds fingers once back in heat

A

become red and stingy

19
Q

primary raynauds

A

happens when young not really likely an auto immune disease

20
Q

causes of secondary raynauds

A

scleroderma
rheumatoid arthritis
SLE
leukemia
use of vibrating tools
contraceptive pill

21
Q

what is raynauds phenomenon

A

secondary raynauds

22
Q

management of raynauds

A

ccb - nifedipine
IV prostacyclin (epoprostenol) infusions: effects may last several weeks/months

23
Q
A